Cure CJD

Heather Larson's experiences in helping find the cure for CJD

Maybe it’s time for genetic counseling: What it is like to be 29 in an E200K genetic mutation family…

Just thinking that I may finally need genetic counseling makes me mad. It’s been nearly five years since my mom died. I did some pretty stupid stuff right after she died but I got over it. I’ve only had one major anxiety attack/PTSD-type moment since, brought on by watching a way-too-realistic seizure scene in the film “Hillary & Jackie.” That hasn’t happened since. I really don’t think I need an overpaid shrink to help me deal with my (alleged) mortality–or at least that’s how I’ve operated for nearly five years. Why get the testing when there is no cure? Why get the testing anyway? I already know my chance is 50/50. If I come out positive, you can just hand me the nails and hammer for the coffin, right? And what if my test results get out somehow and I’m discriminated against despite the anti-genetic discrimination law Bush signed while he was still president? (The only thing he ever did that I believed in).

But lately, my fuse is short as I ponder how to spend what could be my final decade or two. With a mother and uncle who each died at age 56, and me sitting here counting down to my 30th birthday in August…I’m feeling a little pressure. I will also admit I am a complete “Type A” overachiever. So when I ask friends what they’d do with their final decade of life, I didn’t expect to hear the cliches like “I’d live life to the fullest.” I expected people to give me answers like, “I’d get precisely five tattoos, skydive, do two years in the Peace Corps after donating all my possessions to charity, and then I’d climb Everest.”

I’ve been disappointed in my friends’ answers on Facebook and Twitter when I ask my “WWYD” questions. Today, I asked what people would cut out if they had only so many years left. This totally confused people. No one could answer that one. I look at it like this: if my life is to be shorter than even I expected, maybe I cut out waiting in line or sitting in traffic as much as possible. Maybe I pay extra to order stuff online so I don’t have to run errands. Or maybe I figure out how to get by sans car so I never have to drive again. If my life is to be shorter than expected, maybe I cut out wasting it on being a people pleaser or being around people I just can’t stand, even if it’s a neighbor or coworker I don’t want to offend.

These are the kinds of things I think about. I watched a mother die who planned to live forever (her exact quote was, “I’m gonna outlive ’em all!”) After her death I was prepared that the same may happen to my other relatives. I just didn’t think it would be my uncle a mere four years later. I was prepared to walk the road alone but my cousins ended up walking it too. I didn’t think lightning could strike twice like that, but here I am just like that guy in Caddyshack… Which of us will be the next one? Will we even make it to 50? I have noticed younger people are dying of CJD here in the U.S. Look at Eric Bjorkund–he’s only 40! A 54-year-old man in Wisconsin just died of CJD too.

These are some of the reasons for my renewed sense of urgency in confronting any and all mortality-related issues I have. These are issues like how I spend my time, who I spend it with, and do I eat that donut? Do I even bother getting married? Should I even have kids? Who will take care of my cat if/when I die?

Oh, I know what you’re saying. You’re saying, “But Heather, you can get hit by a bus tomorrow. What are you freaking out about?”

To that I say, I’d rather go by getting hit by a bus than by suffering from CJD. You have no idea the things I have seen. But if you follow this blog long enough, I’ll tell you about them. CJD is a horrific way to die. If I have a 50/50 shot of going that way, I want to live the most amazing life possible. Ideally, I wake up tomorrow a rock star opening up a summer music festival arena show with U2, Kings of Leon, Eminem, and Lady Gaga. Then when I’m done with the rock star schtick, I polish my Oscar and pack quickly so I don’t miss my plane to France because it’s time to lay on the beach in Nice. Then when I get back to the U.S., President Obama and the First Lady are inviting me to dinner so we can discuss how to eliminate TSEs from the nation’s food supply. Then we talk about how he can disperse the millions in research money I’ve asked for. In this perfect existence in which I am “living life to the fullest” I am also a size 6 because I really like working out.

Now that you got a laugh, let’s move on…

Think of how the Make A Wish Foundation works. You’re some poor kid who knows she’s going to die, so you tell them you want to meet Steve Carrell so you can die happy. You also want an autograph and cameo role opposite Rainn Wilson on The Office. Poof! Your wish is granted. What would YOU ask the genie for? (Do share, I’ve shared my own pretty silly scenario with you).

Have you read The Last Lecture by Randy Pausch? It’s like that. What would my last lecture be? What would I leave behind to you, besides this blog you’re reading right now? I have the audio book version of this and think all the time about this one story he tells in it. Pausch talks about how the grocery store overcharged him $16 and how he’d rather have the 15 minutes of time in which to go about his day than waste that 15 minutes to get a mere $16 back.


Everything matters that doesn’t matter to normal people my age. Does it matter if I eat the Krispy Kreme? Do I waste 15 minutes cleaning or do I let it go for today so I can call a friend and say hello?


Whether you are Randy Pausch with a year left or me, time matters. I am just like you, really. I haven’t been told I have a terminal illness. I do not know whether I will live to be 40 or 100. But since I have this genetic time bomb ticking in my family, I am constantly thinking about what matters most. What are the top priorities in my life? Do I take risks or get comfy? One friend told me a story about a woman who knew she was going to die, so she made her life as “normal” and routine as possible. These are things I have always shied away from, so I’m not going to start that now.

Like you and everyone else, I have a “bucket list” that is around 2 pages long. I am in a place right now where I cannot deny who I really am or what I really want. If I am unhappy with a person or situation, I walk away. Life is too short! My many “walks away” began shortly after my mom died. But in each decision I have made about how to live my life with fCJD hanging over my head, I have learned a lesson or two. I have found meaning. I have found a better way, happiness, laughter, and sometimes a huge credit card bill.

This is what it’s all about. No matter how long my life may be, I want it to be meaningful. It can’t be wasted or squandered. Do I really have to sleep? Or can I stay up and watch this movie? People who are going to live to be 80 or 90 can go to bed, but maybe I need to stay up. Maybe I do need to take a dance class. Or not. Do I forsake the bucket list in order to just spend time with family, or will that last a week until they start driving me nuts and I go out to buy a jet ski?

It’s like a constant mid-life crisis. If I had the money for it, I’d own a Porsche by now.

Can genetic counseling help me or am I better served by a life coach at this point? I wrote this really long blog post today in hopes it would help give you some insight into what it’s like to be me. My family carries the E200K mutation for familial CJD. Some of us will die of it. Some won’t. Maybe none will–maybe my uncle’s death was the last. Then there’s that river in Africa…

I also wrote this to show you the deep, profound, and lasting effect CJD has had on my life. At age 25 with a dying mother, I did not know this was in my family. I had lived my life in blissful ignorance to that point and I wish I still could. But at age 25, I was also a pompous and selfish ass. I’d so much rather be the person I am today. I earned it.

While sometimes thinking about how CJD affects my life drives me nuts, it is also a hidden blessing. Some of you need the thought of your mortality to put life into perspective for you. In fact, I think some people never get their lives in order until they have a good scare or someone they love dies. There were long periods between my mother’s death and my uncle’s death in which I didn’t think about CJD at all. But what gets me is that I’ve been thinking about my mortality since age 25 in very unique and mature ways. Most people in their 20s don’t have to think like I do. So when I ask you “WWYD” about your final years of life, keep in mind the unique perspective the question comes from. Try for a second to put yourself in my place. Then give me on hell of an interesting answer. Think about it.


June 11, 2009 - Posted by | Uncategorized

No comments yet.

Leave a Reply

Fill in your details below or click an icon to log in: Logo

You are commenting using your account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

%d bloggers like this: